Neuromyelitis Optica Spectrum Disorder and Anti-Aquaporin 4 Channel Immunoglobulin in an Australian Pediatric Demyelination Cohort

2020 ◽  
Vol 35 (4) ◽  
pp. 291-296 ◽  
Author(s):  
Ariel Dahan ◽  
Fabienne Brilot ◽  
Richard Leventer ◽  
Andrew J. Kornberg ◽  
Russell C. Dale ◽  
...  

Neuromyelitis optica spectrum disorder is uncommon in children, and often seronegative for aquaporin-4 immunoglobulin G (AQP4-IgG). We conducted a retrospective study of 67 children presenting to a single Australian center with acquired demyelinating syndromes over a 7-year period. All patients were tested for AQP4-IgG. Five children (7.5%) had neuromyelitis optica spectrum disorder. One child was seropositive for AQP4-IgG (1.5%) and had a relapsing disease course with mild residual deficits. She also had a concomitant motor axonal neuropathy that improved with immunosuppressive therapy. Of the remaining 4 children, 3 had a monophasic course and 1 a relapsing course. Two were tested for anti–myelin oligodendrocyte glycoprotein (anti-MOG) antibody and both were seropositive. This study confirms that neuromyelitis optica spectrum disorder is uncommon in children, and that AQP4-IgG seropositivity is rare. Anti-MOG antibodies should be tested in children with neuromyelitis optica spectrum disorder.

2021 ◽  
Vol 7 (1) ◽  
pp. 10-16
Author(s):  
Vahid Shaygannejad ◽  
◽  
Mahdi Barzegar ◽  
Navid Manouchehri ◽  
Nafiseh Esmaeil ◽  
...  

Background: The absence of Aquaporin-4 Antibody (AQP4-Ab) in a fraction of the Neuromyelitis Optica Spectrum Disorder (NMOSD) patients has led to a search for other serologic markers. Myelin Oligodendrocyte Glycoprotein (MOG) is a protein component of the myelin sheets encapsulating the neural fibers. Objectives: We aimed to compare the presence and levels of anti-MOG (Ig-G) in a group of seronegative NMOSD patients with a healthy control group. Materials & Methods: In this cross-sectional study, 30 NMOSD patients with negative AQP-Ab status, who were referred to the Neurology Clinic of Kashani University Hospital in Isfahan City, Iran, from March 2015 to March 2016, and 26 healthy controls were consecutively recruited. Their baseline demographic and clinical data were recorded. Serum anti-MOG levels were measured in both groups. The obtained data were analyzed using the Student t-test, Mann-Whitney U, and Chisquare test in SPSS V. 18. Results: The anti-MOG test results were statistically higher in patients (n=12, 37.5%) compared to controls (n=0, 0%) (P<0.0001). The level of anti-MOG in Healthy Control (HC) was higher compared to patients with negative anti-MOG (P<0.0001) and was lower than patients with positive anti-MOG (P<0.0001). Conclusion: Our study showed that nearly one-third of seronegative NMOSD patients were positive for MOG-Ab. Further studies are needed to assess the characteristics and outcome of these patients.


2020 ◽  
Vol 22 (2) ◽  
pp. 85-90 ◽  
Author(s):  
Neha Kumar ◽  
Kelsey Graven ◽  
Nancy I. Joseph ◽  
John Johnson ◽  
Scott Fulton ◽  
...  

Abstract Stimulation of the immune response after vaccination can occasionally result in adverse effects, including demyelination of the central nervous system. The most common presentation of postvaccination demyelination is acute disseminated encephalomyelitis, but cases of optic neuritis, transverse myelitis, and multiple sclerosis relapses have been reported. More recently, an increasing number of postvaccination neuromyelitis optica spectrum disorder (NMOSD) cases have surfaced in the literature, especially in patients with aquaporin-4 antibodies. In this article, we report an unusual case of myelin oligodendrocyte glycoprotein antibody–related NMOSD after the receipt of multiple vaccines in a first-trimester pregnant woman from Africa. We review the reported cases of postvaccination demyelination in the past decade, with a focus on the relationship between NMOSD and vaccination in patients with aquaporin-4 or myelin oligodendrocyte glycoprotein antibodies. Finally, we discuss the clinical relevance of the present case and similar reported cases as it relates to patient care in the neuroimmunology clinic and identify potential areas for future research.


2021 ◽  
pp. 44-47
Author(s):  
Cecilia Zivelonghi ◽  
Andrew McKeon

A 12-year-old girl sought care for subacute onset of cramping back pain, along with paresthesias in her lower limbs up to the waistline, both hands, upper back, and chest, followed by rapidly progressive (over a few hours) painful vision loss affecting initially the right eye with subsequent involvement of the left eye. She underwent neuroophthalmologic evaluation and was diagnosed with bilateral optic neuritis. A positive Lhermitte sign was also present. The patient was tested for aquaporin-4-immunoglobulin G autoantibodies, which were positive in both serum and cerebrospinal fluid. A diagnosis of aquaporin-4-immunoglobulin G–positive neuromyelitis optica was made. The patient was treated with rituximab (anti-CD20 monoclonal antibody) and became episode-free, with no further accumulation of disability. The discovery of aquaporin-4-immunoglobulin G in 2004 has permitted the distinction of neuromyelitis optica spectrum disorder from other inflammatory central nervous system disorders. Aquaporin-4-immunoglobulin G represents a highly specific biomarker for neuromyelitis optica (almost 100% using molecular-based techniques), with sensitivity of approximately 80%. According to the most recent diagnostic criteria published in 2015, a diagnosis of neuromyelitis optica spectrum disorder can also be made for patients who are aquaporin-4-immunoglobulin G seronegative by any testing method, regardless of assay sensitivity, provided that more stringent clinical and radiologic requirements are met. Serial testing is recommended for these patients because late seroconversion has been described up to 4 years after the first episode.


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